Case Reports
4 August 2025
Early Access
Rare in the rare: Kikuchi-Fujimoto disease associated with connective tissue disorders. A report of our experience
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All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.
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Kikuchi-Fujimoto disease (KFD) is a rare and benign lymphadenopathy of unknown etiology. Usually, it is an isolated and self-limiting condition requiring no specific therapy; however, in some cases, it may be associated with an autoimmune disease. Here, we report three cases of KFD developing an associated autoimmune connective disorder: the first case presented with Sjögren's syndrome, and the other two had a diagnosis of systemic lupus erythematosus.
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Ahmed Z, Quadir H, Hakobyan K, Gaddam M, Kannan A, Ojinnaka U, et al. Kikuchi-Fujimoto disease: a rare cause of cervical lymphadenopathy. Cureus 2021; 13: e17021. DOI: https://doi.org/10.7759/cureus.17021
Zaccarelli F, de Vincentiis M, D'Erme G, Greco A, Natalucci F, Fusconi M. Kikuchi-Fujimoto disease: a distinct pathological entity but also an "overlap" autoimmune syndrome: a systematic review. Curr Rheumatol Rev 2023; 19: 159-67. DOI: https://doi.org/10.2174/1573397118666220903123128
Chisholm DM, Mason DK. Labial salivary gland biopsy in Sjögren’s disease. J Clin Pathol 1968; 21: 656-60. DOI: https://doi.org/10.1136/jcp.21.5.656
Perry AM, Choi SM. Kikuchi-Fujimoto disease: a review. Arch Pathol Lab Med 2018; 142: 1341-6. DOI: https://doi.org/10.5858/arpa.2018-0219-RA
Rosado FGN, Tang YW, Hasserjian RP, McClain CM, Wang B, Mosse CA. Kikuchi-Fujimoto lymphadenitis: role of parvovirus B-19, Epstein-Barr virus, human herpesvirus 6, and human herpesvirus 8. Hum Pathol 2013; 44: 255-9. DOI: https://doi.org/10.1016/j.humpath.2012.05.016
Aun JA, Hubbard MJ. Kikuchi-Fujimoto disease heralding systemic lupus erythematosus. J Am Osteopath Assoc 2020; 120: 934-9. DOI: https://doi.org/10.7556/jaoa.2020.137
Masab M, Surmachevska N, Farooq H. Kikuchi-Fujimoto Disease. Treasure Island, FL, USA: StatPearls Publishing; 2025.
Dumas G, Prendki V, Haroche J, Amoura Z, Cacoub P, Galicier L, et al. Kikuchi-fujimoto disease: Retrospective study of 91 cases and review of the literature. Medicine 2014; 93: 372-82. DOI: https://doi.org/10.1097/MD.0000000000000220
Cadório MJ, Oliveira J, Gama J, Duarte C. Kikuchi-Fujimoto disease and primary Sjögren's syndrome coexisting: a case-based literature review. Mod Rheumatol Case Rep 2025; 9: 110-6. DOI: https://doi.org/10.1093/mrcr/rxae058
Graef A, Willett A, Dang AH, Balakrishna J, Nicely C, Baiocchi R. A case report: Kikuchi disease associated with a positive auto-immune panel triggered by COVID-19 infection. Cureus 2023; 15: e50911. DOI: https://doi.org/10.7759/cureus.50911
Masiak A, Lass A, Kowalski J, Hajduk A, Zdrojewski Z. Self-limiting COVID-19-associated Kikuchi-Fujimoto disease with heart involvement: case-based review. Rheumatol Int 2022; 42: 341-8. DOI: https://doi.org/10.1007/s00296-021-05088-8
Deaver D, Horna P, Cualing H, Sokol L. Pathogenesis, diagnosis, and management of Kikuchi-Fujimoto disease. Cancer Control 2014; 21: 313-21. DOI: https://doi.org/10.1177/107327481402100407
Yousefi M, Rukerd MRZ, Binafar H, Shoaie S, Mirkamali H, Pourzand P, Kaveh R. The co-occurrence of Kikuchi-Fujimoto disease and systemic lupus erythematosus: a case report. J Med Case Rep 2023; 17: 448. DOI: https://doi.org/10.1186/s13256-023-04186-4
Rana MBM, Fatima M, Rana IM, Haseeb Ul Rasool M, Abosheaishaa H, Abrudescu A, Bandagi S. Kikuchi-Fujimoto disease with systemic lupus erythematosus and systemic sclerosis overlap: a unique clinical presentation. Cureus 2023; 15: e44986. DOI: https://doi.org/10.7759/cureus.44986
Hurtado-Díaz J, Espinoza-Sánchez ML, Rojas-Milán E, Cimé-Aké E, de Los Ángeles Macias M, Romero-Ibarra L, Vera-Lastra OL. Kikuchi-Fujimoto disease: report of a case with progression to lupus nephritis. Am J Case Rep 2021; 22: e927351. DOI: https://doi.org/10.12659/AJCR.927351
Cindy C, Wibowo SAK, Ariane A, Hidayat R. Kikuchi-Fujimoto disease preceding overlap syndrome of Sjögren's syndrome and systemic lupus erythematosus: literature review based on a case report. Acta Med Indones 2024; 56: 210-7.
Wiśniewska K, Pawlak-Buś K, Leszczyński P. Kikuchi-Fujimoto disease associated with primary Sjögren's syndrome - literature review based on a case report. Reumatologia 2020; 58: 251-6. DOI: https://doi.org/10.5114/reum.2020.98438
Gouda W, Alsaqabi F, Almurshed M, Mostafa AA, Albasri A, Negm A, et al. Kikuchi-Fujimoto disease, simultaneously diagnosed with systemic lupus erythematosus in an Arabic female: an agonizing combination. J Int Med Res 2024; 52: 3000605241248884. DOI: https://doi.org/10.1177/03000605241248884
Pham AK, Castillo SA, Barton DT, Rigby WF, Guill MA, Lucas R, LeBlanc RE. Kikuchi-Fujimoto disease preceded by lupus erythematosus panniculitis: do these findings together herald the onset of systemic lupus erythematosus? Dermatol Online J 2020; 26: 13030/qt6tx957m2. DOI: https://doi.org/10.5070/D3268049885
Pradhan S, Sirka CS, Dash G, Sahu K, Rout AN. A challenging case of Kikuchi-Fujimoto disease with systemic lupus erythematosus complicated with hemophagocytic lymphohistiocytosis in a young man. Indian J Dermatol Venereol Leprol 2021; 87: 78-82. DOI: https://doi.org/10.25259/IJDVL_893_18
Amarnani R, Yeoh SA, Denneny EK, Wincup C. Lupus and the lungs: the assessment and management of pulmonary manifestations of systemic lupus erythematosus. Front Med 2020; 7: 610257. DOI: https://doi.org/10.3389/fmed.2020.610257
How to Cite
Rare in the rare: Kikuchi-Fujimoto disease associated with connective tissue disorders. A report of our experience. (2025). Reumatismo. https://doi.org/10.4081/reumatismo.2025.1853
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